Yousaf Muhammad N, Ehsan Hamid, Ehsan Sajid, Sagheer Usman, Chaudhary Fizah
Internal Medicine, MedStar Union Memorial Hospital, Baltimore, USA.
Internal Medicine, MedStar Franklin Square Medical Center, Baltimore, USA.
Cureus. 2020 Jul 21;12(7):e9323. doi: 10.7759/cureus.9323.
Glycogenic hepatopathy (GH) is a rare complication of long-standing uncontrolled type I diabetes mellitus (TIDM) resulting in liver dysfunction and hepatomegaly due to intrahepatic deposition of glycogen. Herein we present a 19-year-old male with a history of TIDM and multiple prior hospitalizations with diabetic ketoacidosis (DKA) who presented with nausea, vomiting, right upper quadrant pain, and massive hepatomegaly. Laboratory workup was consistent with DKA and revealed a greater than 10-fold increase in liver enzymes. Despite the resolution of DKA, his liver function was worsening, and further workup was indicated. Ultimately, he underwent a liver biopsy that showed swollen hepatocytes overloaded with intracytoplasmic glycogen consistent with glycogenic hepatopathy. It is an underestimated entity and physicians should have a high index of suspicion for GH in individuals presenting with liver dysfunction, hepatomegaly, and poor glycemic control in TIDM. Strict glycemic control may result in complete resolution of disease.
糖原性肝病（GH）是长期未得到控制的I型糖尿病（TIDM）的一种罕见并发症，由于肝内糖原沉积导致肝功能障碍和肝肿大。在此，我们报告一名19岁男性，有TIDM病史，曾多次因糖尿病酮症酸中毒（DKA）住院，此次出现恶心、呕吐、右上腹疼痛和肝脏巨大肿大。实验室检查结果与DKA相符，并显示肝酶升高超过10倍。尽管DKA得到缓解，但其肝功能仍在恶化，需要进一步检查。最终，他接受了肝脏活检，结果显示肝细胞肿胀，胞浆内充满糖原，符合糖原性肝病。这是一个被低估的疾病实体，对于TIDM患者出现肝功能障碍、肝肿大和血糖控制不佳的情况，医生应高度怀疑GH。严格的血糖控制可能会使疾病完全缓解。
